Microangiopathic hemolytic anemia: Difference between revisions
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'''Microangiopathic hemolytic anemia (MAHA)''' = variety of conditions that result in damaged endothelium of small vessels causing platelet aggregation and fibrin deposition which leads to shearing forces within these small vessels causing an intravascular fragmentation hemolysis | '''Microangiopathic hemolytic anemia (MAHA)''' = variety of conditions that result in damaged endothelium of small vessels causing platelet aggregation and fibrin deposition which leads to shearing forces within these small vessels causing an intravascular fragmentation hemolysis | ||
== | ==Clinical Features== | ||
*Clinical features + heme labs with red blood cell fragments or schistocytes.<ref name="guidelines">Scully. Marie et al. Guidelines on the diagnosis and management of | *Clinical features + heme labs with red blood cell fragments or schistocytes.<ref name="guidelines">Scully. Marie et al. Guidelines on the diagnosis and management of | ||
thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012 Aug;158(3):323-35. [http://www.bcshguidelines.com/documents/BJH_TTP_Guideline_0512.pdf PDF]</ref> | thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012 Aug;158(3):323-35. [http://www.bcshguidelines.com/documents/BJH_TTP_Guideline_0512.pdf PDF]</ref> | ||
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{{Hemolytic anemia DDX}} | {{Hemolytic anemia DDX}} | ||
== | ==Diagnosis== | ||
*Peripheral smear, hemoglobin, platelets, PT/INR, PTT, fibrinogen, FDPs, D-dimer, haptoglobin, LDH, bilirubin | *Peripheral smear, hemoglobin, platelets, PT/INR, PTT, fibrinogen, FDPs, D-dimer, haptoglobin, LDH, bilirubin | ||
**Schistocytes (aka Helmet cells) secondary to fragmentation hemolysis classic finding on peripheral smear | **Schistocytes (aka Helmet cells) secondary to fragmentation hemolysis classic finding on peripheral smear | ||
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*[[HIT]] | *[[HIT]] | ||
== | ==References== | ||
<references/> | <references/> | ||
[[Category:Heme/Onc]] | [[Category:Heme/Onc]] | ||
Revision as of 13:06, 29 June 2015
Background
Microangiopathic hemolytic anemia (MAHA) = variety of conditions that result in damaged endothelium of small vessels causing platelet aggregation and fibrin deposition which leads to shearing forces within these small vessels causing an intravascular fragmentation hemolysis
Clinical Features
- Clinical features + heme labs with red blood cell fragments or schistocytes.[1]
- May have bleeding and thrombosis
Differential Diagnosis
- Most common causes: DIC, TTP, HUS
- Other medical causes: malignant hypertension, HELLP, Antiphospholipid Syndrome (APS), scleroderma, Paroxysmal Nocturnal Hemoglobinuria (PNH), malignancy, renal allograft rejection, vasculitides like polyarteritis nodosa and Wegener's granulomatosis
- Drugs: chemotherapy; Clopidogrel (Plavix) associated with TTP
- Nonvascular causes: prosthetic valve (more common w/ mechanical, more common at aortic valve), LVAD, TIPS, coil embolization, patched AV shunt, AVM
Microangiopathic Hemolytic Anemia (MAHA)
- Disseminated Intravascular Coagulation (DIC)
- Thrombotic Thrombocytopenic Purpura (TTP)
- Hemolytic Uremic Syndrome (HUS)
- HELLP syndrome
- Heparin-Induced Thrombocytopenia (HIT)
- Hereditary spherocytosis
- Paroxysmal nocturnal hemoglobinuria (PNH)
- Malignant hypertension
- Scleroderma
- Antiphospholipid Syndrome (APS)
- Other medical causes: malignancy, renal allograft rejection, vasculitides like polyarteritis nodosa and Wegener's granulomatosis
- Drugs: chemotherapy; Clopidogrel (Plavix) associated with TTP
- Nonvascular causes: prosthetic valve (more common with mechanical, more common at aortic valve), LVAD, TIPS, coil embolization, patched AV shunt, AVM
Diagnosis
- Peripheral smear, hemoglobin, platelets, PT/INR, PTT, fibrinogen, FDPs, D-dimer, haptoglobin, LDH, bilirubin
- Schistocytes (aka Helmet cells) secondary to fragmentation hemolysis classic finding on peripheral smear
Lab Findings
- Schistocytes
- High: +/- LDH and unconjugated bilirubin
- Low: Hgb, platelets, +/- haptoglobin
General Rules
Management
See Also
- DIC
- TTP
- HUS
- Malignant hypertension, HELLP, Antiphospholipid Syndrome (APS), Paroxysmal Nocturnal Hemoglobinuria (PNH)
- HIT